The establishment of national diagnostic reference levels for adult SPECT-CT in Saudi Arabia.

This study aims to introduce national diagnostic reference levels (NDRLs) for adult hybrid single photon emission computed tomography (SPECT-CT) in nuclear medicine (NM) departments in the Kingdom of Saudi Arabia. The administered activity (AA) of radiopharmaceuticals, volume-weighted computed tomography dose index (CTDIvol) and dose length product (DLP) for ten hybrid SPECT/CT examinations were collected and analysed for one year. The median of AA, CTDIvoland DLP for each dose quantity was derived and the suggested national DRLs were determined based on the 75thpercentile for all identified SPECT-CT examinations. A comparison of the defined adult NDRLs in Saudi Arabia with the published data of other countries was performed. Although there are no significant variations of the proposed NDRL of AA between countries, the proposed NDRLs of the integrated CT metrics exceed the published data in most procedures. NM departments are urged to consider optimisation for both image quality and radiation protection.

Stressors for emergency department nurses: Insights from a cross-sectional survey.

AIMS AND OBJECTIVES: This study aimed to profile stress, exploring whether demographics and/or other factors uniquely contribute to stress for emergency departments nurses. BACKGROUND: Nursing has been identified as a stressful profession with the potential to have negative impacts on nurses' physical and mental health. Nurses working in emergency departments face unique daily stressors. DESIGN: Cross-sectional survey using a correlational design. METHODS: The survey was distributed in May 2019 to ED nurses working in New South Wales (NSW), Australia. A total of 242 nurses completed the survey, of which 190 cases were analysed. A standardised scale was used in the survey, the Expanded Nursing Stress Scale (ENSS). The sampling method for this research was non-probability purposive sampling. STROBE checklist was used for this manuscript. RESULTS: The results of this study indicated that emergency department nurses experience stress in their work settings. Stress is experienced predominantly because of problems in dealing with patients and their families, high workloads, and uncertainty concerning treatment. CONCLUSIONS: The results of this study provide valuable insights into the work-related stress experienced by emergency department nurses. This information can be used to inform the development of strategies to minimise stress for emergency nurses. RELEVANCE TO CLINICAL PRACTICE: This study builds on existing, and contributes to new, knowledge about the current stressors for emergency department nurses. The results provide insights into how policies and procedures may need to be adjusted and interventions developed, that can help in reducing stress.

Use of intraoperative intracavitary (direct-contact) ultrasound for resection control in transsphenoidal surgery for pituitary tumors: evaluation of a microsurgical series.

BACKGROUND: Perisellar infiltration may be responsible for incomplete removal of pituitary tumors. Since intraoperative visualization of parasellar structures is difficult during transsphenoidal surgery, we are describing the use of intraoperative direct contact ultrasound (IOUS). METHODS: Within 5 years, in 113 transsphenoidal operations (58 male, 55 female, age 14-81 years, 110 pituitary adenomas (mean diameter 26.6 mm, 69 non-secreting adenomas, 41 secreting adenomas), and 1 of each Rathke's cleft cyst, craniopharyngioma, and xanthogranuloma), IOUS was applied. After wide opening of the sellar floor and removal of the intrasellar tumor portions, a commercially available side fire ultrasound probe is introduced, and in direct contact to the sellar envelope, the perisellar space is scanned perpendicular to the axis of the working channel. We compared the results of IOUS to postoperative MRI after 3-6 months. RESULTS: Identification of the intracavernous ICA, the anterior optic pathway, and the ACA, was possible, it was safe to operate close to them. In 65 operations (58%), further resection of tumor remnants was performed after IOUS. In this selected series, complete resection of tumors (stated by postoperative MRI after 3-6 months) was achieved in 75 operations (66%) and remission was achieved in 18 operations of secreting adenomas (44%). Compared to MRI after 3 to 6 months, the sensitivity of IOUS was 0.568 and the specificity was 0.907. No complications related to IOUS were seen. CONCLUSIONS: Visualization of the perisellar compartments by IOUS is easy and fast to perform. It allows the surgeon to identify resectable tumor remnants intraoperatively, which otherwise could be missed.

High-dose Chemotherapy With Autologous Stem Cell Rescue in Saudi Children Less Than 3 Years of Age With Embryonal Brain Tumors.

High-dose chemotherapy with autologous stem cell rescue (HDC/ASCR) has been used in children under the age of 3 years with embryonal brain tumors to avoid or delay the use of radiation. We reviewed the medical records of 10 Saudi children less than 3 years of age with embryonal brain tumors who underwent HDC/ASCR. All 10 patients underwent surgical resection followed by 3 to 5 cycles of induction chemotherapy and 1 to 3 cycles of HDC/ASCR using carboplatin and thiotepa. Isotretinoin was used as a maintenance therapy in 4 patients. Five patients had medulloblastoma, 3 had atypical teratoid/rhabdoid tumors, 1 had an embryonal tumor with abundant neuropil and true rosettes, and 1 had pineoblastoma. The median age of the patients was 1.9 years. A total of 19 HDC/ASCR procedures were performed. Radiotherapy (RT) was administered to 5 patients after HDC/ASCR and as a salvage therapy in 1 patient. The progression-free survival rate was 50% at 1 year and at 2 years, with a median follow-up of 24 months. All 5 patients with medulloblastoma are still alive without evidence of disease, but the other patients died secondary to tumor progression. This experience suggests that strategies combining myeloablative chemotherapy and autologous stem cell rescue appear to be feasible for children with embryonal brain tumors in the Middle East.

Shunt Dependence after Intraventricular Hemorrhage and Intraventricular Fibrinolysis with uPA versus rt-PA.

BACKGROUND: We compare the effect of urokinase (urokinase-type plasminogen activator [uPA]) versus alteplase (recombinant tissue plasminogen activator [rt-PA]) for intraventricular fibrinolysis (IVF) in patients with intraventricular hemorrhage (IVH) on ventriculoperitoneal shunt (VPS) dependence, functional outcome, and complications in the management of IVH. METHODS: We retrospectively reviewed the patients admitted with IVH or intracerebral hemorrhage (ICH) with IVH within 7 years in three different departments and found 102 patients who met the inclusion criteria. The primary end points were VPS dependence and Glasgow outcome score (GOS) at 3 months. Secondary end points were rate of rebleeding under IVF and incidence of treatment-related complications. Patients were divided into three groups: group I comprised patients treated with external ventricular drain (EVD) and IVF with uPA; group II comprised patients treated with EVD and IVF with rt-PA; and group III comprised patients treated with EVD alone. RESULTS: In all, 9.8% patients needed VPS: 12.2% in group I and 15.0% in group II, with no statistically significant difference. VPS patients had higher values of the modified Graeb score (mGS), IVH score, and IVH volume. We saw a trend for a better outcome in group II, with six patients achieving a GOS of 4 or 5 after 3 months. The mortality rate was higher in groups I and III. We found no statistical difference in the complication rate between groups I and II. Logistic regression analysis revealed that higher mGS and age predicted worse prognosis concerning mortality. The risk for death rose by 7.8% for each year of age. Any additional mGS point increased the chances of death by 9.7%. CONCLUSION: Our data suggest that both uPA and rt-PA are safe and comparable regarding incidence of communicating hydrocephalus, and age and mGS are predictive for mortality.

Predictive Value of Early Postoperative Course of Serum Cortisol After Transsphenoidal Surgery for Cushing's Disease.

OBJECTIVE: To identify early available predictors for the long-term outcome of patients after transsphenoidal surgery (TSS) in the management of Cushing's disease. METHODS: This single-center, retrospective study included 93 consecutive patients with Cushing's disease (follow-up 12-129 months, mean 48, median 38) who underwent TSS (21 had previous operations elsewhere). Six cases had early re-operation, and the resulting data were evaluated instead of the respective first operation. During the postoperative course, serum cortisol levels were assessed every four hours at least until the next morning. An association of parameters with long-term outcomes was tested using binary logistic regression. Receiver operating characteristic curves were used to determine sensitivity, specificity, positive predictive value, and negative predictive value of different cut-off values of serum cortisol in the postoperative course in the event of recurrence after remission. RESULTS: Eighty out of 93 patients (86%) showed postoperative remission (after primary treatment, 60 out of 72 patients, 90.3%). Of these, 8 patients (10%) developed recurrence of hypercortisolism. Compared to patients with persisting long-term remission, those with recurrence differed in cortisol levels starting from 4 pm on the day of surgery plus an event of increasing cortisol during the early postoperative course ("peak"). Binary logistic regression showed the association between a peak of serum cortisol in the early postoperative course with an increased probability of recurrence. CONCLUSIONS: Patients with a peak of serum cortisol in the early postoperative course show an increased recurrence rate. A cut-off value of serum cortisol for clear identification of patients with later recurrence could not be determined.

Rhinological Consequences of Microsurgical Endonasal-Transsphenoidal Surgery for Pituitary Tumors.

BACKGROUND: Transsphenoidal surgery for sellar lesions may affect patency and function of the nasal airways, smell and sinonasal quality of life. Below is our report on otorhinolaryngological data garnered from patients undergoing endonasal transsphenoidal pituitary microsurgery. METHODS: In a prospective study, 68 patients scheduled for transsphenoidal operations (32 female, 36 male, age 17-72 years) underwent otorhinolaryngological evaluation of their nasal morphology, a standardized smell test (sniffin' sticks) and rhinomanometry to analyse nasal breathing function preoperatively, 3-5 days postoperatively (without rhinomanometry), after 3-4 months and after 9 months. RESULTS: Immediately after surgery, a reduction in smell sensation was detected in almost all patients. Within 3 months, this impairment resolved in all cases except one. In 2 patients (3%) with preoperative anosmia, improvement of smell function to>6 out of 12 sniffin' sticks was observed. At final visit no patient was noted to have new anosmia. Within 3 months, the results of the rhinomanometry revealed that all patients except one, regained their preoperative nasal breathing function. In 6 patients (8.8%) an improvement in their nose breathing abilities compared to the preoperative state was found. Three patients (4.4%) underwent a LASER transection of mucosal synechiae. In one case with persistent nasal obstruction (1.5%), secondary septoplasty had to be performed. There was no case in which perforation of the nasal septum, nasal tip deflection, or saddle nose deformity was observed. CONCLUSION: Microsurgical resection of pituitary tumors via the endonasal transsphenoidal approach poses an acceptable risk with regards to sinonasal complications. The incidence of secondary rhinosurgical interventions is low. Standardized comparative studies between endoscopic and microsurgical transsphenoidal operations should be undertaken.

Clinical Characteristics and Outcomes of Pediatric Patients With Immune Thrombocytopenic Purpura in King Abdulaziz Medical City and King Abdullah Specialist Children's Hospital: A 10-Year Study.

Background Immune thrombocytopenic purpura (ITP) can be defined as "an immune-mediated acquired disease characterized by a transient or persistent decrease in the platelet count". Medical treatment is usually not needed but, in some cases, intravenous immunoglobulin G (IVIG), corticosteroids, and anti-D immunoglobulins are used. Splenectomy can be an option for chronic cases with no response to pharmacological treatments. The aim of this study was to describe the clinical characteristics and outcomes of pediatric patients with ITP in King Abdulaziz Medical City (KAMC) and King Abdullah Specialist Children's Hospital (KASCH) in a 10-year period. Methods The study was conducted at KAMC and KASCH. The number of recorded cases was 95, which included all ITP patients aged 1 to 14 from both genders who presented to KAMC previously and KASCH currently from January 1, 2007, to December 31, 2017. The data analysis and entry were performed using the Statistical Package for the Social Sciences (SPSS) version 25 (IBM Corp., Armonk, NY). Results Among 95 pediatric patients with ITP, 51 (53.7%) were males and 44 (46.3%) were females, with a median age of 4.00 ± 3.977. Among them, 84 (92.7%) had purpura, 38 (46.3%) had epistaxis, 43 (39%) had petechia, 17 (24.3%) had fever, and 20 (19.5%) had gum bleeding. Out of 95 patients, 91 (95.8%) were given treatment. Out of those 91 patients who were given treatment, IVIG was used in 84 (92.3%), steroids were used in 44 (48.4%), 14 patients received platelet transfusion (15.4%), rituximab was used in 7 (7.7%), and splenectomy was done in 5 (5.5%); 32 (33.7%) cases were considered chronic (more than one year), and 63 (66.3%) were considered acute. Among chronic patients, only one death was recorded, while in acute, no deaths were recorded. Conclusion In conclusion, ITP is an autoimmune disease that decreases platelet count. The results showed a significant difference in treatment compared to the literature but similar results in other aspects.

Bone volume fraction and structural parameters for estimation of mechanical stiffness and failure load of human cancellous bone samples; in-vitro comparison of ultrasound transit time spectroscopy and X-ray µCT.

Conventional mechanical testing is the 'gold standard' for assessing the stiffness (N mm-1) and strength (MPa) of bone, although it is not applicable in-vivo since it is inherently invasive and destructive. The mechanical integrity of a bone is determined by its quantity and quality; being related primarily to bone density and structure respectively. Several non-destructive, non-invasive, in-vivo techniques have been developed and clinically implemented to estimate bone density, both areal (dual-energy X-ray absorptiometry (DXA)) and volumetric (quantitative computed tomography (QCT)). Quantitative ultrasound (QUS) parameters of velocity and attenuation are dependent upon both bone quantity and bone quality, although it has not been possible to date to transpose one particular QUS parameter into separate estimates of quantity and quality. It has recently been shown that ultrasound transit time spectroscopy (UTTS) may provide an accurate estimate of bone density and hence quantity. We hypothesised that UTTS also has the potential to provide an estimate of bone structure and hence quality. In this in-vitro study, 16 human femoral bone samples were tested utilising three techniques; UTTS, micro computed tomography (µCT), and mechanical testing. UTTS was utilised to estimate bone volume fraction (BV/TV) and two novel structural parameters, inter-quartile range of the derived transit time (UTTS-IQR) and the transit time of maximum proportion of sonic-rays (TTMP). µCT was utilised to derive BV/TV along with several bone structure parameters. A destructive mechanical test was utilised to measure the stiffness and strength (failure load) of the bone samples. BV/TV was calculated from the derived transit time spectrum (TTS); the correlation coefficient (R2) with µCT-BV/TV was 0.885. For predicting mechanical stiffness and strength, BV/TV derived by both µCT and UTTS provided the strongest correlation with mechanical stiffness (R2=0.567 and 0.618 respectively) and mechanical strength (R2=0.747 and 0.736 respectively). When respective structural parameters were incorporated to BV/TV, multiple regression analysis indicated that none of the µCT histomorphometric parameters could improve the prediction of mechanical stiffness and strength, while for UTTS, adding TTMP to BV/TV increased the prediction of mechanical stiffness to R2=0.711 and strength to R2=0.827. It is therefore envisaged that UTTS may have the ability to estimate BV/TV along with providing an improved prediction of osteoporotic fracture risk, within routine clinical practice in the future.

The dependence of broadband ultrasound attenuation on phase interference in thin plates of variable thickness and curvature: a comparison of experimental measurement and computer simulation.

The measurement of broadband ultrasound attenuation describes the linear increase in ultrasound attenuation with frequency (dB/MHz); this is generally performed at the calcaneus, consisting of a high proportion of metabolically active cancellous bone. Although broadband ultrasound attenuation is not routinely implemented within clinical management since it cannot provide a reliable estimation of bone mineral density and hence clinical definition of osteopenia and osteoporosis, it offers a reliable means to predict osteoporotic fracture risk. One of the potential factors that can influence the accuracy of broadband ultrasound attenuation measurement is the effect of cortical end plates. This study aimed to explore this, performing a comparison of experimental study and computer simulation prediction. A total of three categories of thin discs were three-dimensional (3D) printed to replicate cortical shells of (1) variable constant thickness (planar), (2) variable constant thickness (curved), and (3) variable thickness. A through-transmission technique was used, where two single-element, unfocused, 1 MHz broadband transducers, as utilised clinically, were positioned coaxially in a cylindrical holder and immersed in water. Both quantitative and qualitative analyses demonstrated that broadband ultrasound attenuation measurements of the 'planar' and 'curved' discs were not statistically different (p-values > 0.01). A cyclic relationship between broadband ultrasound attenuation and disc thickness was observed; this was replicated within a computer simulation of phase interference created by a double-reflection echo within each disc (R2 = 97.0%). Variable-thickness discs provided broadband ultrasound attenuation measurements ranging between 31.6 ± 0.1 and 40.60 ± 0.1 dB/MHz. Again applying the double-reflection echo simulation, a high level of agreement between experimental and simulation was recorded (R2 = 93.4%). This study indicates that the cortical end plate can significantly affect the broadband ultrasound attenuation measurement of cancellous bone as a result of phase interference and, therefore, warrants further investigation to minimise its effect on clinical assessment.

Ectopic Adrenocorticotropic Hormone-Secreting Pituitary Adenomas: An Underestimated Entity.

BACKGROUND: The diagnosis of Cushing disease is based on endocrinological pa-rameters, with no single test being specific. In some patients, dynamic thin-slice sellar magnetic resonance imaging fails to detect a pituitary tumor. OBJECTIVE: The purpose of this study is to investigate the role of ectopic pituitary adenoma in this situation. METHODS: In a retrospective chart review, 5 patients (6%) with ectopic adenomas were identified in 83 consecutive patients undergoing transsphenoidal surgery for adrenocorticotropic hormone (ACTH)-secreting pituitary adenomas by 1 surgeon. RESULTS: In all 5 patients (all female, 32-41 years of age), an exclusively extrasellar ACTH-secreting adenoma was excised. Three adenomas were located in the cavernous sinus, 1 in the sphenoid sinus, and 1 in the ethmoidal cells. Histologically, none of the tumors showed signs of aggressiveness. Three of the 5 adenomas specifically expressed somatostatin receptor 5. In 4 patients with Cushing disease, postoperative remission was obtained, with 1 recurrence after 14 months. In the patient with Nelson syndrome, ACTH decreased from >800 to <80 pg/mL. Three patients underwent previous surgery elsewhere, including 1 hypophysectomy. In this case, the ectopic adenoma (positive for somatostatin receptor 5) in the ethmoidal cells turned out to be positive on gallium 68 DOTATATE positron emission tomography/computed tomography. CONCLUSION: The incidence of primarily ectopic ACTH-secreting adenomas in this series was 6%. In cases of negative MRI findings, an ectopic ACTH-secreting adenoma should be taken into account. 68 Ga DOTATATE positron emission tomography/computed tomography may identify ectopic pituitary adenomas. Hypophysectomy should always be avoided in primary surgery for CD.

Foreign body aspiration in children: A study of children who lived or died following aspiration.

Foreign body aspiration (FBA) is a preventable cause of mortality and morbidity in children. We conducted a chart review of children who presented to a university hospital due to FBA in the period 1999-2014. Children were either managed with bronchoscopy for removal of the foreign body or died due to FBA. A total of 103 children were seen due to FBA including 27 deaths. The majority of children were boys and were less than 3 years old. Most aspirated foreign bodies were food-related, mainly peanuts. The majority of children presented with acute choking incidents, a smaller number presented with recurrent chest infections, and few children's choking incidents were unwitnessed. X-ray had a high rate of false negatives and bronchoscopy was the gold standard technique for assessment and management. Aspiration of foreign bodies is a preventable, life-threatening condition that calls for increased parent education and awareness.

The prevalence of factor VIII and IX inhibitors among Saudi patients with hemophilia: Results from the Saudi national hemophilia screening program.

Hemophilia A and B are X-linked diseases that predominantly affect male patients. Patients can develop coagulation factor inhibitors, which exponentially increases the treatment cost. However, the prevalence of factor VIII and IX inhibitors in Saudi Arabia is unclear.This study aimed to determine the Saudi prevalence of factor VIII and IX inhibitors.This 4-year, 7-center, cross-sectional study evaluated the Saudi prevalences of hemophilia A and B. We collected the patients' clinical data, evaluated their disease, and tested for factor inhibitors.We included 202 patients with hemophilia (median age at diagnosis: 0.13 years, range: birth-34.8 years). The patients included 198 male patients (98%), 148 patients with hemophilia A (73.3%), and 54 patients with hemophilia B (26.7%). The patients exhibited severe factor VIII activity (<1%; 121 patients; 5.2%), moderate activity (1-5%; 7 patients; 4.9%), and mild activity (14 patients; 9.9%). Among the patients with care-related data, most patients were treated for episodic bleeding (76.8%) or received prophylaxis (22.6%); 1 patient received both treatments. Among the patients with source-related data, the factor replacements were derived from plasma (48.4%), recombinant concentrates (22.9%), both sources (14.6%), or fresh frozen plasma (14.1%). Factor VIII inhibitors were observed in 43 (29.3%) of the 147 patients, and only 1 of the 54 patients developed factor IX inhibitors. Most patients who developed inhibitors had severe hemophilia (40/44; 90.9%), and inhibitors were also common among patients who received recombinant products (14/43; 32.6%).The Saudi prevalence of factor inhibitors was similar to those among other ethnic populations.

Intracardiac Leakage of Cement During Kyphoplasty and Vertebroplasty: A Case Report.

BACKGROUND: Intracardiac leakage of bone cement after kyphoplasty and vertebroplasty is a rare and life-threatening complication. Cortoss, which is an injectable, non-absorbable, polymer composite that is designed to mimic cortical bone, can be used instead of cement. Here, we present the case of a patient with right intra-cardiac Cortoss embolization. CASE REPORT: A 28-year-old man known to have ulcerative colitis since the age of 15 and treated with corticosteroids for more than 4 years and with anti-immune drugs presented to our hospital complaining of back pain and decreased body height due to osteomalacia with failed conservative treatment. Kyphoplasty and vertebroplasty of the thoracic 10-12 and first lumbar vertebrae were done with any complications. Three months later, the patient underwent kyphoplasty and vertebroplasty of lumbar 2-5 vertebrae by injecting Cortoss instead of cement, which was complicated with paravertebral intravascular leakage. We stopped surgery and transferred him to the recovery room, where he had slight chest pain that resolved spontaneously without neurological deficit. Two days later he developed severe chest pain and chest X-ray showed a large white shadow at the right side of the heart and another 2 small shadows just lateral to it. Sudden deterioration of patient status necessitated an emergency echocardiogram, which showed pericardial tamponade and a perforated right ventricle. Aspiration of pericardial blood and emergency open heart surgery were done. He was discharged 4 days later and was followed up at an outpatient clinic. CONCLUSIONS: Cardiac embolism is a serious condition that can complicate vertebral kyphoplasty; it requires a high level of suspicion and immediate action, and may need open heart surgery to save the patient's life.

Clinical characteristics and genetic subtypes of Fanconi anemia in Saudi patients.

We reviewed our institutional experience from 2011 to 2015 on new cases of Fanconi anemia (FA). Ten unrelated cases were diagnosed during this period. Four patients with severe aplastic anemia (SAA) had c.2392C > T (p.Arg798*) BRIP1/FANCJ mutation. Another child with SAA had novel c.1475T > C (p.Leu492Pro) FANCC mutation. One individual with SAA and acute myeloid leukemia had c.637_643del (p.Tyr213Lysfs*6) FANCG mutation. Three patients presented with early onset of cancer, two had BRCA2 mutation c.7007G > A (p.Arg2336His) and one had a novel c.3425del (p.Leu1142Tyrfs*21) PALB2 mutation. Another infant with c.3425del PALB2 mutation had clonal aberration with partial trisomy of the long arm of chromosome 17. Mutations in FA downstream pathway genes are more frequent in our series than expected. Our preliminary observation will be confirmed in a large multi-institutional study.

Comparison if the addition of multilevel vertebral augmentation to conventional therapy will improve the outcome of patients with multiple myeloma.

BACKGROUND: This was a prospective study to evaluate the effect of multilevel vertebral augmentation in addition to conventional therapy in multiple myeloma patients. METHODS: We treated 27 patients, whom were recently diagnosed to have multiple myeloma by two ways of treatment. Thirteen patients (group I) were treated with conventional therapy and 14 patients (group II) with adding vertebroplasty and kyphoplasty. Patients were evaluated pre-treatment and at half, one, two and 3-years post-treatment by using Oswestry Disability Index (ODI), the Stanford Score (SS) and the Spinal Instability Neoplastic Score (SINS). RESULTS: Mean values of ODI, SS and SINS were 31.9 (63.8%), 4.3 and 13.8 for group I and 33.2 (66.4%), 4.6 and 12.8 for group II before starting treatment. Group II showed improvement better than group I at all follow-up intervals with best results at first 6 months. P-values at the end of the study were ODI = 0.047, SS = 0.180 and SINS = 0.002. Mortality rates were equal of both groups (four patients of each group). CONCLUSION: Adding vertebral augmentation to conventional therapy improves multiple myeloma patients' quality of life, but didn't affect the mortality rate.